| Identification | Back Directory | [Name]
1-Propanaminium, 3-carboxy-N,N,N-trimethyl-2-(2-methyl-1-oxobutoxy)-, inner salt, (2R)- | [CAS]
256928-75-3 | [Synonyms]
2-Methylbutyryl-L-carnitine >=97.0% (TLC)
3-((2-Methylbutanoyl)oxy)-4-(trimethylammonio)butanoate
1-Propanaminium, 3-carboxy-N,N,N-trimethyl-2-(2-methyl-1-oxobutoxy)-, inner salt, (2R)- | [Molecular Formula]
C12H23NO4 | [MDL Number]
MFCD11975299 | [MOL File]
256928-75-3.mol | [Molecular Weight]
245.32 |
| Chemical Properties | Back Directory | [storage temp. ]
2-8°C | [form ]
Solid | [color ]
White to off-white | [Optical Rotation]
[α]/D -20±2°, c =0.1 in H2O | [InChI]
1S/C12H23NO4/c1-6-9(2)12(16)17-10(7-11(14)15)8-13(3,4)5/h9-10H,6-8H2,1-5H3/t9?,10-/m1/s1 | [InChIKey]
IHCPDBBYTYJYIL-QVDQXJPCSA-N | [SMILES]
C[N+](C)(C)C[C@H](OC(C(CC)C)=O)CC([O-])=O |
| Hazard Information | Back Directory | [Uses]
2-Methylbutyrylcarnitineis was detected in a study of maternal liver metabolic response to vitamin D deficiency. | [Definition]
ChEBI: 2-methylbutyrylcarnitine is a C5-acylcarnitine having 2-methylbutyryl as the acyl substituent. It has a role as a human metabolite. It is functionally related to a 2-methylbutyric acid. | [Biological Activity]
2-Methylbutyryl-L-carnitine is not usually detected in normal individuals. The elevation of 2-methylbutyrylcarnitine suggests a deficiency of a dehydrogenase specific for isobutyryl-CoAimportant in the differential diagnosis of branched chain organic acidurias by analysis of urinary organic acids and acylcarnitines in plasma or dried blood spots. |
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